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• W1991332286 abstract "In this report, we review the recent advances in evaluation and treatment of transfusional iron overload (IO). Results of the French thalassaemia registry are described. According to the disease, thalassaemia major or sickle cell anaemia, mechanisms and toxicity of iron overload, knowledge about IO long-term outcome and chelation treatment results, respective value of IO markers, differ. The recent tools evaluating organ specific IO and the diversification of iron chelator agents make possible to individualize chelation therapy in clinical practice. The severity of IO and the level of transfusional iron intake, the preferential localization of IO (heart/liver) as well as the tolerance and adherence profiles of the patient can now be taken into account. Introduction of cardiac magnetic resonance imaging for the quantification of myocardial iron and use of oral chelators have already been reported as decreasing the cardiac mortality rate related to IO in thalassaemia major patients. Long-term observation of patients under oral chelators will show if morbidity is also improving via a more continuous control of toxic iron and/or a better accessibility to cellular iron pools. Nous rapportons les progrès majeurs réalisés dans le domaine de l’évaluation et du traitement de la surcharge en fer post-transfusionnelle au cours des dernières années, ainsi que les principaux résultats de la prise en charge des patients atteints de formes sévères de bêta-thalassémie résidant en France et répertoriés dans le registre national. La surcharge en fer consécutive aux transfusions régulières de concentrés globulaires concerne principalement deux maladies constitutionnelles de l’hémoglobine : la thalassémie et la drépanocytose. Concernant la surcharge en fer accompagnant ces deux hémoglobinopathies, elle diffère dans ses mécanismes, la fréquence de ses complications cliniques, le niveau de connaissance sur son pronostic à long terme, la documentation des bénéfices de son traitement et la pertinence de ses indicateurs paracliniques. Les nouveaux outils d’imagerie par résonance magnétique évaluant la surcharge en fer tissulaire et la diversification de l’arsenal thérapeutique avec l’utilisation des chélateurs du fer actifs par voie orale ont déjà permit une amélioration de l’espérance de vie des patients thalassémiques. Le traitement chélateur peut être individualisé car adapté à la localisation prédominante (cardiaque et/ou hépatique) et à la sévérité de la surcharge ainsi qu’au profil de tolérance et d’observance du patient." @default.
• W1991332286 created "2016-06-24" @default.
• W1991332286 creator A5059187541 @default.
• W1991332286 date "2013-03-01" @default.
• W1991332286 modified "2023-10-14" @default.
• W1991332286 title "Post-transfusional iron overload in the haemoglobinopathies" @default.
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• W1991332286 doi "https://doi.org/10.1016/j.crvi.2012.09.010" @default.
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